 |
|
|
|
|
|
 |
 |
 |
 |
 |
 |
|
|||||||||||||||||||
Title: |
Unique Transgenic Animal Model for Hereditary Hearing Loss | ||||||||||||||||||
| Authors: | Maura Cosetti, MD; David Culang, MD; Sumankrishna Kotla, MD; Peter O‘Brien; Daniel F. Eberl, PhD; Frances Hannan, PhD | ||||||||||||||||||
|
Objectives: This study capitalizes on the unique molecular and developmental similarities between the auditory organs
of Drosophila and mammals, to investigate genes implicated in human syndromic and nonsyndromic hearing loss in a
genetically tractable experimental animal model, the fruit fly Drosophila.
Methods: The Drosophila counterparts of 3 human deafness genes (DIAPH1/DFNA1, ESPN/DFNB36, and TMHS/DFNB67) were identified by sequence similarity. An electrophysiological assay was used to record sound-evoked potentials in response to an acoustic stimulus, the Drosophila courtship song. Results: Flies with mutations affecting the diaphanous, forked, and CG12026/TMHS genes displayed significant reductions in the amplitude of sound-evoked potentials compared to wild-type flies (p < 0.05 to p < 0.005). The mean responses were reduced from approximately 500 to 600 μV in wild-type flies to approximately 100 to 300 μV in most mutant flies. Conclusions: The identification of significant auditory dysfunction in Drosophila orthologs of human deafness genes will facilitate exploration of the molecular biochemistry of auditory mechanosensation. This may eventually allow for novel diagnostic and therapeutic approaches to human hereditary hearing loss. (Ann Otol Rhinol Laryngol 2008;117:827-833.) |
|||||||||||||||||||
| Keywords: | auditory evoked response, deafness mutant, Drosophila, microfilament protein, tetraspan membrane protein | ||||||||||||||||||
| Acrobat Reader 8.0 is recommeded to properly view and print the article.
Reader can be downloaded here:  |
|||||||||||||||||||
